Outcome of Staged Reconstructive Surgery for Hypoplastic Left Heart Syndrome Following Antenatal Diagnosis

Overview

Journal Arch Dis Child

Specialty Pediatrics

Date 2001 Nov 24

PMID 11719331

Citations 9

Authors

R Andrews

R Tulloh

G Sharland

J Simpson

S Rollings

E Baker

S Qureshi

E Rosenthal

C Austin

D Anderson

Affiliations

Soon will be listed here.

Abstract

Background And Aims: Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis allows for appropriate counselling, and time to consider treatment options. We report outcome from a centre where most cases are antenatally diagnosed and delivered on site.

Methods: Information was collated on 188 consecutive cases of HLHS between 1995 and 2000, including timing of diagnosis, outcome of pregnancy, and age and outcome at each stage of surgery. At Guy's Hospital, 174 cases were diagnosed antenatally, of whom 50 underwent surgery. Fourteen others (five diagnosed antenatally at other centres, and nine diagnosed postnatally) also underwent surgery.

Results: Survival after stage I (the Norwood operation) was 52% (33/64). Postoperative survival after stage II (the hemi-Fontan operation, performed in 29), and stage III (the Fontan operation, performed in 10), was 100%. Two late deaths occurred 3 and 10 months after stage II, giving overall survival of 48% (31/64). At follow up, three children have neurological impairment, and one had poor right ventricular function necessitating cardiac transplantation.

Conclusions: Antenatal diagnosis allows informed decisions about treatment options, and facilitates preoperative care. Mortality following stage I is high, irrespective of timing of diagnosis, but medium term outcome for survivors is good.

Citing Articles

Medium-term Outcome of Prenatally Diagnosed Hypoplastic Left-Heart Syndrome and Impact of a Restrictive Atrial Septum Diagnosed in-utero.

Ramcharan T, Quintero D, Stickley J, Poole E, Miller P, Desai T Pediatr Cardiol. 2023; 44(6):1217-1225.

PMID: 37219587 DOI: 10.1007/s00246-023-03184-z.

Current state of the art in hypoplastic left heart syndrome.

Birla A, Brimmer S, Short W, Olutoye 2nd O, Shar J, Lalwani S Front Cardiovasc Med. 2022; 9:878266.

PMID: 36386362 PMC: 9651920. DOI: 10.3389/fcvm.2022.878266.

Hypoplastic left heart syndrome [HLHS]: treatment options in present era.

Rai V, Gladki M, Dudynska M, Skalski J Indian J Thorac Cardiovasc Surg. 2020; 35(2):196-202.

PMID: 33061005 PMC: 7525540. DOI: 10.1007/s12055-018-0742-z.

Hypoplastic left heart syndrome, cor triatriatum and partial anomalous pulmonary venous connection: Imaging of a very rare association.

Khan M, Almoukirish A, Das K, Galal M J Saudi Heart Assoc. 2013; 24(2):137-40.

PMID: 23960683 PMC: 3727436. DOI: 10.1016/j.jsha.2011.10.003.

Hypoplastic left heart syndrome.

Connor J, Thiagarajan R Orphanet J Rare Dis. 2007; 2:23.

PMID: 17498282 PMC: 1877799. DOI: 10.1186/1750-1172-2-23.

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